Practical guidelines for the diagnosis and treatment of transthyretin amyloid cardiomyopathy (ATTR-CM or transthyretin cardiac amyloidosis)

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Abstract

This paper summarizes the data from updated international protocols and guidelines for diagnosis of transthyretin amyloid cardiomyopathy (ATTR-CM). The invasive and non-invasive diagnosis techniques and their combinations are briefly reviewed; the evidentiary foundations for each diagnostic option and tool are analyzed. The paper describes a customized algorithm for sequential diagnosis and differential diagnosis of patients with suspected ATTR-CM with allowance for the combination of clinical signs and diagnostic findings. Along with the awareness of primary care providers about the red flags of the disease and visualization criteria, as well as providing information to the patients about the possibility of performing therapy of ATTR amyloidosis and the risks of delayed diagnosis, the proposed algorithm enables timely patient routing and prescribing specific treatment.

About the authors

Sergey N. Tereshchenko

Chazov National Medical Research Center of Cardiology; Russian Medical Academy of Continuous Professional Education

Email: izhirov@mail.ru
ORCID iD: 0000-0001-9234-6129

д-р мед. наук, проф., рук. отд. заболеваний миокарда и сердечной недостаточности, зав. каф. кардиологии 

Russian Federation, Moscow; Moscow

Igor V. Zhirov

Chazov National Medical Research Center of Cardiology; Russian Medical Academy of Continuous Professional Education

Author for correspondence.
Email: izhirov@mail.ru
ORCID iD: 0000-0002-4066-2661

д-р мед. наук, вед. науч. сотр., проф. каф. кардиологии

Russian Federation, Moscow; Moscow

Olga M. Moiseeva

Almazov National Medical Research Centre; Pavlov First Saint Petersburg State Medical University

Email: izhirov@mail.ru
ORCID iD: 0000-0002-7817-3847

д-р мед. наук, дир. Института сердца и сосудов, рук. отд. некоронарогенных заболеваний сердца, проф. каф. факультетской терапии 

Russian Federation, Saint Petersburg; Saint Petersburg

Tatiana V. Adasheva

Yevdokimov Moscow State University of Medicine and Dentistry

Email: izhirov@mail.ru
ORCID iD: 0000-0002-3763-8994

д-р мед. наук, проф. каф. поликлинической терапии, чл. президиума РНМОТ

Russian Federation, Moscow

Alexey A. Ansheles

Chazov National Medical Research Center of Cardiology

Email: izhirov@mail.ru
ORCID iD: 0000-0002-2675-3276

д-р мед. наук, вед. науч. сотр. отд. радионуклидной диагностики и позитронно-эмиссионной томографии

Russian Federation, Moscow

Olga L. Barbarash

Research Institute of Complex Problems of Cardiovascular Diseases; Kemerovo State Medical University

Email: izhirov@mail.ru
ORCID iD: 0000-0002-4642-3610

чл.-кор. РАН, д-р мед. наук, проф., дир., зав. каф. кардиологии

Russian Federation, Kemerovo; Kemerovo

Albert S. Galyavich

Kazan State Medical University

Email: izhirov@mail.ru
ORCID iD: 0000-0002-4510-6197

д-р мед. наук, проф., зав. каф. кардиологии фак-та повышения квалификации и профессиональной переподготовки специалистов

Russian Federation, Kazan

Alexandra Ia. Gudkova

Almazov National Medical Research Centre; Pavlov First Saint Petersburg State Medical University

Email: izhirov@mail.ru
ORCID iD: 0000-0003-0156-8821

д-р мед. наук, вед. науч. сотр. Института молекулярной биологии и генетики, зав. лаб. кардиомиопатий НИИ сердечно- сосудистых заболеваний Научно-клинического исследовательского центра, проф. каф. факультетской терапии

Russian Federation, Saint Petersburg; Saint Petersburg

Dmitry A. Zateyshchikov

City Clinical Hospital №51; Central State Medical Academy of the President of the Russian Federation

Email: izhirov@mail.ru
ORCID iD: 0000-0001-7065-2045

д-р мед. наук, проф., зав. первичным сосудистым отд-нием, зав. каф. терапии, кардиологии и функциональной диагностики с курсом нефрологии

Russian Federation, Moscow; Moscow

Anna A. Kostareva

Almazov National Medical Research Centre

Email: izhirov@mail.ru
ORCID iD: 0000-0002-9349-6257

д-р мед. наук, дир. Института молекулярной биологии и генетики

Russian Federation, Saint Petersburg

Svetlana N. Nasonova

Chazov National Medical Research Center of Cardiology

Email: izhirov@mail.ru
ORCID iD: 0000-0002-0920-7417

канд. мед. наук, ст. науч. сотр. отд. заболеваний миокарда и сердечной недостаточности НИИ клинической кардиологии им. А.Л. Мясникова

Russian Federation, Moscow

Sergey V. Nedogoda

Volgograd State Medical University

Email: izhirov@mail.ru
ORCID iD: 0000-0001-5981-1754

д-р мед. наук, проф., зав. каф. внутренних болезней Института непрерывного медицинского и фармацевтического образования

Russian Federation, Volgograd

Tamara B. Pecherina

Research Institute of Complex Problems of Cardiovascular Diseases

Email: izhirov@mail.ru
ORCID iD: 0000-0002-4771-484X

канд. мед. наук, доц., ст. науч. сотр. лаб. патологии кровообращения отд. клинической кардиологии

Russian Federation, Kemerovo

Daria V. Ryzhkova

Almazov National Medical Research Centre

Email: d_ryjkova@mail.ru
ORCID iD: 0000-0002-7086-9153

д-р мед. наук, проф., врач-радиолог высшей категории, гл. науч. сотр. научно-исследовательского отд. ядерной медицины и тераностики Института онкологии и гематологии, рук. научно-клинического объединения ядерной медицины, зав. каф. ядерной медицины и радиационных технологий Института медицинского образования

Russian Federation, Saint Petersburg

Vladimir B. Sergienko

Chazov National Medical Research Center of Cardiology

Email: izhirov@mail.ru
ORCID iD: 0000-0002-0487-6902

д-р мед. наук, проф., рук. отд. радионуклидной диагностики и позитронно-эмиссионной томографии

Russian Federation, Moscow

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2. Fig. 1. Evaluation of the absorption of an osteotropic radiopharmaceutical by the myocardium during scintigraphy with phosphate complexes labeled with 99mTc. Grade 0: no myocardial uptake of the radiopharmaceutical and normal uptake by bone tissue; grade 1: absorption by the myocardium to a lesser extent than by the bone tissue; grade 2: similar myocardial and bone uptake; and grade 3: myocardial uptake is greater than bone uptake or no bone uptake.

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3. Fig. 2. Diagnostic algorithm transthyretin amyloid cardiomyopathy.

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